Cavalli Tiziana, Giudici Francesco, Nesi Gabriella, Amorosi Andrea, Santi Raffaella, Brandi Maria Luisa, Tonelli Francesco
Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50134, Florence, Italy.
Institute of Pathology, Department of Health Sciences, University "Magna Graecia" of Catanzaro, Catanzaro, Italy.
Fam Cancer. 2017 Apr;16(2):249-256. doi: 10.1007/s10689-016-9936-y.
Approximately 300 cases of sporadic parathyroid cyst (PCs) have been reported to date. Only two cases have been described in MEN1 so far. Detection by imaging could be challenging, especially in multiglandular primary hyperparathyroidism (HPT) and clinical outcome could be different. During the period 1990-2014, 71 MEN1 patients were operated for primary hyperparathyroidism in our centre. We report three cases of PCs in MEN1 patients affected by HPT, who underwent a total or subtotal parathyroidectomy with transcervical thymectomy. In our series, all three patients had an unsatisfactory postoperative course, at variance with the high percentage (over 90 %) of long-term success in MEN1 patients operated at our centre. One patient affected by cystic degeneration of all the four parathyroid glands reported persistent hypoparathyroidism, despite autografts of parathyroid tissue. For the other two cases, surgery failed to cure hyperparathyroidism, perhaps because of the presence of undetected ectopic parathyroid tissue. In the context of a multiglandular disease such as MEN1 syndrome, PCs seem rare but our experience shows about a 4 % incidence. Furthermore their presence, even in expert hands, could affect the preoperative identification of the parathyroid glands due to the difficult differential diagnosis between PC and other cystic lesions of the neck, and intraoperative detection of the glands as well as the postoperative outcome.
迄今为止，大约已报道了300例散发性甲状旁腺囊肿（PCs）。到目前为止，在多发性内分泌腺瘤1型（MEN1）中仅描述了2例。通过影像学进行检测可能具有挑战性，尤其是在多腺体原发性甲状旁腺功能亢进症（HPT）中，而且临床结果可能有所不同。在1990年至2014年期间，我们中心有71例MEN1患者因原发性甲状旁腺功能亢进症接受了手术。我们报告了3例受HPT影响的MEN1患者中的PCs病例，这些患者接受了甲状旁腺全切或次全切除术并经颈部胸腺切除术。在我们的系列病例中，所有3例患者术后病程均不理想，这与在我们中心接受手术的MEN1患者长期成功率较高（超过90%）形成反差。1例所有4个甲状旁腺均发生囊性变的患者，尽管进行了甲状旁腺组织自体移植，但仍报告有持续性甲状旁腺功能减退。对于另外2例，手术未能治愈甲状旁腺功能亢进症，可能是因为存在未被发现的异位甲状旁腺组织。在诸如MEN1综合征这样的多腺体疾病背景下，PCs似乎很少见，但我们的经验显示其发生率约为4%。此外，由于PC与颈部其他囊性病变之间的鉴别诊断困难，其存在即使在经验丰富的医生手中，也可能影响甲状旁腺的术前识别、术中腺体的检测以及术后结果。
